scleroderma
TRANSCRIPT
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SCLERODERMA
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Systemic sclerosis Also called scleroderma Multisystem disease of unknown etiology Pathogenesis include immune system
activation , endothelial activation , and fibroblast activation
this results in small blood vessels damage and tissue fibrosis.
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Epidemiology Rare disease incidence in the US 19/million/year
and prevalence of 19-75/100,000 Rare in children Peak between age 35-65 More women affected Family h/o other auto immune diseases Ethnic background influence survival and
disease manifestation.
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Clinical features Raynaud’s Typical skin changes Esophageal and small bowel dysfunction Interstitial lung disease Pulmonary hypertension Renal crises
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What is Raynaud’s Phenomenon Reversible skin color changes:White to blue to
red Due to vasospasm Induced by cold of emotion
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Raynaud’s :Pallor phase
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Raynaud’s:cyanotic phase
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Causes of Raynaud’s Occlusive arterial disease Rheumatic
diseases:Scleroderma,CREST,MCTD,SLE,RA, Myositis
Repetitive vascular injury Hyperviscosity :Polycythemia,Cryoglobulinemia Thoracic outlet syndrome
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Typical skin changes Tight thick skin,peaked nosePursed mouth
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Salt and pepper pigmentation
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Microstomia
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Digital ulcers
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Esophageal dysmotility:heart burn,and reflux symptoms Dilated esophagus onEsophagogram
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CREST Calcinosis Raynaud’s Esophageal dysmotility Sclerodactyly Telangiectasias
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Calcinosis
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Sclerodactyly Skin changes,and flexion contactures
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Telangiectasias Dilated capillaries seenover the face
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Renal crises and hypertension in scleroderma Major complication Early in disease first few years Acute onset hypertension High Renin Renal impairment Microangiopathic hemolytic anemia,Thrombocytopenia Renal failure can be reversible if BP treated Drug of choice Angiotensin converting enzyme inhibitor
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Shortness of breath in Scleroderma DD,1-Interstitial lung disease:CXR,PFT,HRCT chest2-Pulmonary hypertension:Echocardiogram,right
sided cardiac catheterization3-Cardiomyopathy,Heart failure,4-Pericardial effusion5-Renal crises
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Interstitial Lung disease Major cause of morbidly
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Diagnosis Clinical features ANA positive 90% Anti topoisomerase 1 antibody positive(scl-70)
30% in diffuse Anticentromere antibody positive in CREST and
limited scleroderma
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Treatment Skin :no effective treatment,60% improve with
time Treat hypertension early and aggressive to
prevent renal damage and other hypertension complications
Calcium channel blockers may help Raynaud’s Treat ILD and pulmonary hypertension Avoid steroids(may provoke renal complications)